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MND Association/RCGP motor neurone disease guideline

Motor neurone disease - red flag diagnosis tool

The GP’s role in motor neurone disease (MND) is pivotal, not just in coordinating services, but also in the prompt management of symptoms, which can generally improve quality of life

How does amyotrophic lateral sclerosis/motor neurone disease present?

Presentation is commonly in two patterns: either ‘spinal’ (75%) with weakness in one or more limbs, or ‘bulbar’ with speech and swallowing problems (25%)

The earliest symptoms may be non-specific such as muscle cramps or persistent muscle twitching (fasciculation). However it should be remembered that both symptoms are frequently a normal phenomenon in healthy people, and fasciculation tends to be less intrusive to MND patients. There may be insidious ‘clumsiness’ and/or falls, which go unreported

The recognition of progressive weakness without sensory involvement is the key to prompting the consideration of MND as a diagnosis

What differential diagnoses need to be considered?

Other diseases which lead to muscular wasting, especially of the upper limbs, should be considered. They are (in approximate order of frequency):

cervical spondylosis

inclusion body myositis

myasthenia gravis

multi-focal motor neuropathy with conduction block (MMN/CB)

cervical rib

peripheral nerve lesions

syringomyelia

intramedullary spinal tumour

polymyositis

What is the role of the multi-disciplinary team (MDT)?

The optimal care of MND patients and their families/carers is best delivered by a well co-ordinated multi-disciplinary team approach:

physiotherapists have expertise in relation to mobility and posture

early referral to speech and language therapy services at the first suggestion of bulbar dysfunction is important

prompt referral to a dietitian is advised if there is concern regarding inadequate caloric intake. Weight loss has a significant adverse effect on survival

Referral

1. Does the patient have one or more of these symptoms?

Bulbar features:

dysarthria

slurred or quiet speech often when tired

dysphagia

liquids and/or solids

excessive saliva

choking sensation especially when lying flat

tongue fasciculations

Limb features:

focal weakness

falls/trips—from foot drop

loss of dexterity

muscle wasting

muscle twitching/fasciculations

cramps

no sensory features

Respiratory features:

hard to explain respiratory symptoms

shortness of breath on exertion

excessive daytime sleepiness

fatigue

early morning headache

orthopnoea

Cognitive features (may not be a presenting feature):

behavioural change

emotional lability (not related to dementia)

fronto-temporal dementia

2. Is there progression?

Supporting factors:

asymmetrical features

age—MND can present at any age

positive family history of MND or other neurodegenerative disease

Factors NOT supportive of MND diagnosis

bladder/bowel involvement

prominent sensory symptoms

double vision/ptosis

improving symptoms

If yes to 1 and 2, query MND and refer to Neurology

If you think it might be MND please state explicitly in the referral letter

Common causes of delay are initial referral to ENT or orthopaedic services

The RCGP accredited version of the Red Flag diagnosis tool along with an accompanying presentation are available to download from www.mndassociation.org/redflag

Useful sources of information

The Motor Neurone Disease Association in England, Wales, and Northern Ireland, and the Scottish MND Association provide support to patients and their carers on all aspects of MND, along with information packs for GPs—see www.mndassociation.org and www.mndscotland.org.uk for MND Scotland